Sequeira Cristiana, Coelho Mariana, Costa Santos Inês, Ramos Lopes Sara, Mangualde João, Oliveira Ana Paula
Gastroenterology Department, Centro Hospitalar de Setúbal, Setúbal, Portugal.
GE Port J Gastroenterol. 2022 Oct 31;30(5):398-402. doi: 10.1159/000526854. eCollection 2023 Oct.
Cholestatic liver diseases may be associated with increased plasmatic cholesterol due to an abnormal lipoprotein - lipoprotein X (LpX). Correcting the underlying cause of cholestasis is the critical treatment of LpX-associated hypercholesterolemia without any proven benefit from conventional lipid-lowering agents. In some situations, plasma exchange may apply to prevent associated complications, such as hyperviscosity syndrome. The authors present the case of a 44-year-old man with orbital inflammatory pseudotumor on prednisolone, admitted due to hepatocellular and cholestatic lesion and severe hypercholesterolemia. Laboratory investigation established that hepatitis E virus was responsible for liver injury and showed that LpX mediated the severe hypercholesterolemia. Reduction of the immunosuppressive load contributed to virus clearance. The consequent resolution of cholestasis and cholesterol removal by plasmapheresis allowed lipid profile normalization. The authors report the first case of LpX-associated hypercholesterolemia in a patient with hepatitis E-induced cholestasis and revisit the role of the liver in lipid metabolism.
胆汁淤积性肝病可能与血浆胆固醇升高有关，这是由于异常脂蛋白——脂蛋白X（LpX）所致。纠正胆汁淤积的根本原因是治疗LpX相关高胆固醇血症的关键，而传统降脂药物并无已证实的益处。在某些情况下，血浆置换可用于预防相关并发症，如高黏滞综合征。作者报告了一例44岁男性患者，因眼眶炎性假瘤服用泼尼松龙，因肝细胞和胆汁淤积性病变及严重高胆固醇血症入院。实验室检查确定戊型肝炎病毒是肝损伤的病因，并表明LpX介导了严重的高胆固醇血症。降低免疫抑制负荷有助于病毒清除。随后胆汁淤积的缓解以及通过血浆置换清除胆固醇使血脂谱恢复正常。作者报告了首例戊型肝炎引起胆汁淤积患者发生LpX相关高胆固醇血症的病例，并重新审视了肝脏在脂质代谢中的作用。
